Fertility prospects for the prune-belly patient: A scoping review.

INTRODUCTION: With advances in medical care and assisted reproductive technologies (ART), fertility prospects for prune-belly syndrome (PBS) men may be changing. This review aims to identify the factors influencing fertility and optimization of reproductive health for PBS patients. MATERIAL AND METHODS: A scoping review was performed on all records published over 70 years (1952-2022) analyzing fertility in PBS males. Records were summarized in a table and narrative describing cryptorchidism, orchiopexy, testicle histology; prostate characteristics; sex hormone function; semen analyses, ART, and conception ability. This review was registered on Open Science Framework (OSF) and conducted using PRISMA methodology. RESULTS: 827 articles were identified and 83 were selected for data extraction. Before 2000, there were 0.85 publications/year whereas after 2000 there were 1.95 publications/year. Orchiopexy successfully relocated 86 % of PBS testicles into the scrotum. Testicular histology demonstrated 50 % of patients had no spermatogonia, while 47.2 % and 2.7 % had reduced or normal numbers respectively. Leydig hyperplasia and Sertoli only histology were found in 19.4 % of patients. Prostatic hypoplasia and prostatic urethral dilation were found in 93.6 % and 91.4 % of patients respectively. Testosterone, Luteinizing hormone (LH) and Follicle-stimulating hormone (FSH) were normal in 93.9 %, 87.7 % and 77.9 % of patients respectively. Azoospermia and oligospermia was found in 75.7 % and 21.6 % of patients respectively while 60.7 % had antegrade ejaculation. ART successfully extracted sperm in 6 instances and resulted in 4 conceptions, while natural conception was reported twice. CONCLUSIONS: Data analysis indicates increased attention to fertility prospects for PBS males with evaluation of PBS patient's hormonal function, semen analyses, ART, and conception ability. The reviewed data suggest that PBS males may father biological offspring with contemporary management and also demonstrate the need for consistent reproductive management approaches to maximize their fertility prospects.

Medium and Long-Term Clinical Outcomes of Kidney Transplantation in Patients With Prune Belly Syndrome: A Single-Centre Experience.

OBJECTIVE: To present the medium and long-term outcomes of kidney transplantation performed in patients with Prune Belly Syndrome (PBS) which is one of the congenital anomalies of the kidney and urinary tract (CAKUT). METHODS: Thirteen kidney transplantations were performed in nine patients with PBS from January 1994 to December 2020. Demographics, surgical features, graft and patient survival rates were analyzed. Survival outcomes was compared with 106 age- and donor-type matched controls who underwent transplantation because of nonurological causes. RESULTS: The median ages of PBS patients at the time of first and second transplantation were 19 and 34 years old, respectively. One patient had postoperative Clavien grade 3a complication. Eight of 13 transplants are still functional at the last follow-up. Eight patients underwent pretransplant urological operations in preparation for kidney transplantation, while 4 patients underwent urological operations in the post-transplant period. Graft survival for PBS transplants at 5, 10, and 15 years were 90.9%, 57.7%, and 38.5%, respectively. Graft survival for control cohort at 5, 10, and 15 years were 89.9%, 80%, and 74%, respectively. The patient and graft survival rates were similar between PBS and control groups (P = .449, P = .134, respectively). CONCLUSION: This single-center study showed that the medium-term graft survival rates in patients with PBS are encouraging and comparable to non-CAKUT transplant patients, however long-term graft survival rates were found to be proportionally inferior. To help determine the reasons for long-term transplant kidney function deterioration and how to improve them we suggest that multicenter international collaboration will be needed.

Surgical approach to prune-belly syndrome: A review of our series and novel surgical technique.

INTRODUCTION: Surgical management of children with Prune-belly syndrome (PBS) can be divided into three categories: urinary tract reconstruction, abdominal wall reconstruction, and orchidopexy. Adequate repair of the abdominal wall by abdominoplasty at an early age, allows an adequate aesthetic appearance, but also allows a correct development of walking, breathing, defecation and urination. OBJECTIVE: To present a novel surgical technique for abdominal wall reconstruction, which combines plication with complete overlap of the fascia and neoumbilicoplasty with an island flap rotated on itself. STUDY DESIGN: A retrospective review of the patients with PBS who underwent surgery in our center between 2009 and 2020. A new abdominoplasty technique was performed, which consists of plication with complete overlap of the fascia, as well as a neoumbilicoplasty with an island flap rotated on itself. The skin is then well freed from the rest of planes, and the plication is performed with complete overlapping of the muscle-aponeurotic plane from one side to the other. At this time, the interposition of some type of reinforcement material (mesh) may be required in the event of complete absence or insufficient presence of the fascia and muscle. RESULTS: Abdominoplasty was performed in four patients. In all patients, bilateral orchidopexy was performed in the same surgical procedure for intra-abdominal testicles. The mean age of the patients at the time of surgery was 10,5 years and the average admission time was 4 days. There were no short-term or long-term complications. Average follow-up time was 7 years. All patients and their families are satisfied with the aesthetic result obtained. DISCUSSSION: Abdominoplasty in patients with PBS in addition to aesthetic reconstruction, brings significant improvements to wandering, breathing, urination and defecation, improving the quality of life of the patient. Compared to the techniques described, the abdominoplasty we propose makes it possible to easily improve the abdominal wall support by means of meshes or synthetic devices if necessary and allows the evaluation and correction of cryptorchidism or other associated renal abnormalities in the same surgical act, as it allows full abdominal exposure. Likewise our modified abdominoplasty allows the creation of the new umbilicus in a more anatomical way, rectifying its natural position. CONCLUSION: In our experience, this modified surgical technique for abdominal wall repair is a novel procedure in the PBS approach, easily reproducible, which provides good aesthetic results in our series of cases.

Modern management of and update on prune belly syndrome.

Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, a variable degree of urinary tract involvement and cryptorchidism. Most cases of PBS are sporadic and have a normal karyotype, with 95% patients being male. In the last decade, mutations in known genes that regulate embryonic genitourinary myogenesis have been identified and with increasing knowledge of these critical genes involved in bladder maldevelopment, advances can be made in genetic counseling. A multidisciplinary approach is necessary and individualization of care is recommended according to phenotypic severity. Some patients require abdominal and urinary tract reconstruction while others require as little as bilateral orchiopexies. Major treatment objectives are: preservation of renal function and upper urinary tract; polyuria management; adequate bladder emptying; improvement of corporal image and quality of life; preservation of fertility and adequate sexual function. Long-term surveillance of the urinary tract is essential up to adulthood, because functional dynamics can change over time.

A novel technique of abdominoplasty for prune belly syndrome.

Prune belly syndrome is an extremely rare congenital condition occurring predominantly in males. This triad syndrome comprises of partial or complete deficient abdominal wall muscles, undescended testes and dilated urinary collecting system. We present the case of a 2-year-old male patient, who presented with classic prune belly syndrome, operated with modification in classical Monfort technique of abdominoplasty. The basis of this approach was to save and use the supraumbilical abdominal wall which has better tensile strength. The infraumbilical skin was mostly discarded. The outcome was better in terms of cosmetic appearance as the scar was limited to the lower abdomen and better anterior abdominal wall strength.

Repercusión de la pandemia COVID-19 sobre la actividad quirúrgica de Urología Pediátrica: análisis de las complicaciones posquirúrgicas según la clasificación de Clavien-Dindo / Impact of the COVID-19 pandemic on the surgical activity of Pediatric Urology: analysis of postoperative complications according to the Clavien-Dindo classification

INTRODUCCIÓN Y OBJETIVO: La coronavirus disease 2019 (COVID-19) ha ocasionado una pandemia de repercusión mundial que obligó a tomar medidas sociopolíticas, como la declaración del estado de alarma en España. Paralelamente se llevó a cabo la reestructuración de las actividades e infraestructuras médico-quirúrgicas pediátricas, con la consecuente suspensión de la actividad quirúrgica no urgente de Urología Pediátrica. Analizamos la repercusión de la pandemia COVID-19 sobre la actividad quirúrgica en una sección de Urología Pediátrica, así como las complicaciones quirúrgicas, según la clasificación de Clavien-Dindo. MATERIALES Y MÉTODOS: Se procedió a la revisión sistemática de los datos epidemiológicos, clínicos y quirúrgicos, incluyendo las complicaciones y reingresos de todos los pacientes intervenidos en la sección de Urología Pediátrica desde la declaración del estado de alarma hasta el levantamiento del mismo. Para su estudio se procedió a la división en cinco bloques temporales acorde a las fases de desescalada. RESULTADOS: Se realizaron 49 intervenciones quirúrgicas en 45 pacientes (ocho previos a la implantación de las fases de desescalada). La patología con prioridad alta fue la más frecuente en las primeras fases, siendo la estenosis de la unión pieloureteral (EPU) la más prevalente. Se registraron cuatro complicaciones (8,8%), ninguna de ellas de origen respiratorio. CONCLUSIONES: Las recomendaciones de la EAU para la reanudación de la actividad quirúrgica han permitido una correcta, segura y gradual transición al ritmo quirúrgico habitual en Urología Pediátrica. La clasificación de Clavien-Dindo es útil y válida para su aplicación en esta sección. No se han registrados complicaciones respiratorias que pudiesen ser atribuibles a la situación pandémica

INTRODUCTION AND OBJECTIVE: The coronavirus disease 2019 (COVID-19) has caused a pandemic of global impact that forced social-political measures to be taken, such as the declaration of the state of alarm in Spain. At the same time, the reorganization of the pediatric medical-surgical activities and infrastructures was carried out, with the consequent suspension of the non-urgent surgical activity of Pediatric Urology. We analyzed the impact of the COVID-19 pandemic on surgical activity in a Pediatric Urology division, as well as surgical complications according to the Clavien-Dindo classification. MATERIALS AND METHODS: A systematic review of epidemiological, clinical and surgical data was carried out, including complications and readmissions of all patients operated on in the division of Pediatric Urology within the duration of the state of alarm. Five time periods have been created according to the de-escalation phases. RESULTS: Forty-nine surgical procedures were carried out on 45 patients (8 prior to the implementation of the de-escalation phases). High priority pathologies were the most frequent in the first phases, being the ureteropelvic junction (UPJ) obstruction the most prevalent. Four complications were recorded (8.8%), none of them were respiratory. CONCLUSIONS: The EAU recommendations for the resumption of surgical activity have allowed a correct, safe and gradual transition to the routine surgical activity in Pediatric Urology. The Clavien-Dindo classification is useful and valid for application in this division. No respiratory complications have been reported that could be attributable to the pandemic situation

Impact of the COVID-19 pandemic on the surgical activity of Pediatric Urology: analysis of postoperative complications according to the Clavien-Dindo classification. / Repercusión de la pandemia COVID-19 sobre la actividad quirúrgica de Urología Pediátrica: análisis de las complicaciones posquirúrgicas según la clasificación de Clavien-Dindo.

INTRODUCTION AND OBJECTIVE: The coronavirus disease 2019 (COVID-19) has caused a pandemic of global impact that forced social-political measures to be taken, such as the declaration of the state of alarm in Spain. At the same time, the reorganization of the pediatric medical-surgical activities and infrastructures was carried out, with the consequent suspension of the non-urgent surgical activity of Pediatric Urology. We analyzed the impact of the COVID-19 pandemic on surgical activity in a Pediatric Urology division, as well as surgical complications according to the Clavien-Dindo classification. MATERIALS AND METHODS: A systematic review of epidemiological, clinical and surgical data was carried out, including complications and readmissions of all patients operated on in the division of Pediatric Urology within the duration of the state of alarm. Five time periods have been created according to the de-escalation phases. RESULTS: Forty-nine surgical procedures were carried out on 45 patients (8 prior to the implementation of the de-escalation phases). High priority pathologies were the most frequent in the first phases, being the ureteropelvic junction (UPJ) obstruction the most prevalent. Four complications were recorded (8.8%), none of them were respiratory. CONCLUSIONS: The EAU recommendations for the resumption of surgical activity have allowed a correct, safe and gradual transition to the routine surgical activity in Pediatric Urology. The Clavien-Dindo classification is useful and valid for application in this division. No respiratory complications have been reported that could be attributable to the pandemic situation.

Minimal Invasive Linea Alba Reconstruction for the Treatment of Umbilical and Epigastric Hernias with Coexisting Rectus Abdominis Diastasis.

INTRODUCTION: Patients with umbilical or epigastric hernias benefit from mesh- based repairs, and even more so if a concomitant rectus diastasis (RD) is present. The ideal technique is, however, still under debate. In this study we introduce the minimal invasive linea alba reconstruction (MILAR) with the supraaponeurotic placement of a fully absorbable synthetic mesh. MATERIALS AND METHODS: Midline reconstruction with anterior rectus sheath repair and mesh augmentation by an open approach is a well-known surgical technique for ventral hernia repair. Between December 1, 2016, and November 30, 2017, 20 patients with symptomatic umbilical and/or epigastric hernias, and coexisting RD underwent a minimally invasive complete reconstruction of the midline through a small access route. The inner part of both incised and medialized anterior rectus sheaths was replaced by a fully absorbable synthetic mesh placed in a supraaponeurotic position. RESULTS: Patients were hospitalized for an average of 4 days and the mean operating time was 79 minutes. The mean hernia defect size was 1.5 cm in diameter and the mean mesh size was recorded as 15.8 cm in length and 5.2 cm in width. Two patients sustained surgical postoperative complications in terms of symptomatic seroma occurrences with successful interventional treatment.The early results (mean follow-up period of 5 months) showed no recurrences and only 1 patient reported occasional pain following exertion without rest. DISCUSSION: MILAR is a modification of the recently published endoscopic linea alba reconstruction restoring the normal anatomy of the abdominal wall. A new linea alba is formed with augmentation of autologous tissue consisting of the plicated anterior rectus sheaths. Supraaponeurotic placement of a fully absorbable synthetic mesh eliminates potential long-term mesh-associated complications. Regarding MILAR, there is no need for endoscopic equipment due to the uniquely designed flexible lighted retractors, meaning one assistant less is required.

Combined laparoscopic and open technique for repair of congenital abdominal hernia: A case report of prune belly syndrome.

BACKGROUND: Prune belly syndrome (PBS) is a rare congenital disorder among adults, and the way for repairing abdominal wall musculature has no unified standard. MATERIALS AND METHODS: We described combining laparoscopic and open technique in an adult male who presented with PBS. Physical examination and radiological imaging verified the case of PBS. The deficiency of abdominal wall musculature was repaired by combining laparoscopic and open technique using a double-deck complex patch. RESULTS: The patient successfully underwent abdominal wall repair by combining laparoscopic and open technique. Postoperative recovery was uneventful, and improvement in symptom was significant in follow-up after 3, 6, 12, and 24 months. CONCLUSIONS: Combining laparoscopic and open technique for repair of deficiency of abdominal wall musculature in PBS was an exploratory way to improve life quality.

Abdominoplasty in prune belly syndrome: Modifications in Monfort technique to address variable patterns of abdominal wall weakness.

INTRODUCTION: Abdominoplasty is an important component of the management of children with prune belly syndrome (PBS). While there are features of the abdominal defect in PBS which are common to all patients, there will be differences unique to each patient that should be taken into consideration in surgical planning. Specifically, we have come to realize that although the Monfort procedure assumes a symmetric pattern of abdominal wall laxity, this symmetry is rarely present. OBJECTIVE: The aim of this report is to describe our modifications and review our outcomes for the Monfort procedure which more completely address correction of the abdominal wall laxity including both common and uncommon features while positioning the umbilicus to a more anatomically correct position (Figure). STUDY DESIGN: Sixteen male patients with PBS and one female pseudoprune belly syndrome patient, aged 2-9 years, were treated at our institution between 2003 and 2014. Modifications incorporated into the abdominoplasty procedure for PBS applied to this study group included: 1) use of diagnostic laparoscopy to define the topography of the abdominal wall defect, 2) initial midline rather than elliptical skin incision to defer retailoring of the skin coverage until the final step of the procedure, 3) varying the width of the central plate to correct side to side asymmetry in redundancy, 4) plication of the central plate to reduce vertical redundancy and reposition the umbilicus, and 5) plication of focal areas of fascial weakness, most often in the flank region. RESULTS: All patients have improved abdominal wall contour with a more uniform correction of areas of weakness at a mean follow-up of 5.5 years (range 18 months-11.5 years). All patients and parents indicate that they are very satisfied with the outcome of their procedures without any revisions being performed. This study is descriptive in nature and retrospective, with the patient population treated in a relatively uniform fashion that does not allow direct comparison with other techniques. CONCLUSIONS: The modified Monfort procedure recognizes the pattern of abdominal muscular deficiency unique to each patient and incorporates this information into the surgical design.

Proposed technique for open repair of a small umbilical hernia and rectus divarication with self-gripping mesh.

INTRODUCTION: There are a group of patients in which umbilical or epigastric hernias co-exist with rectus divarication. These patients have weak abdominal musculature and are likely to pose a higher risk of recurrence following umbilical hernia repair. We would like to describe a technique for open repair of small (<4 cm) midline hernias in patients with co-existing rectus divarication using self-adhesive synthetic mesh. The use of a self-adhesive mesh avoids the need for suture fixation of the mesh in the superior portion of the abdomen, allowing for a smaller skin incision. RESULTS: In 173 patients, preperitoneal self-fixating mesh has been used for the repair of midline hernias <4 cm in diameter. In 58 of these patients, the mesh was extended superiorly to reinforce a concurrent divarication. DISCUSSION: The described technique offers a simple option for open repair of small midline hernias in patients with co-existing rectus divarication, to decrease the risk of upper midline recurrence in an at-risk patient group. This initial case series is able to demonstrate a suitably low rate of recurrence and complications.

Abdominoplasty in Prune Belly Syndrome.

INTRODUCTION: Many patients with Prune Belly Syndrome (PBS) require abdominoplasty alone or in combination with correction of any urogenital abnormalities. This video presents a simplified technique with which to treat the abdominal flaccidity in PBS. METHODS: A longitudinal xypho-pubic fusiform figure is drawn on the abdomen, based on the area of skin and subcutaneous tissue to be removed. This is performed with preservation of the musculo-fascial layer and the umbilicus. A lateral elliptical single xypho-pubic line is drawn in the most lax side of the fascia, which is incised along this line. After urinary tract reconstruction and orchidopexy, closure is initiated by suturing the medial edge of the wider fascial flap laterally to the peritoneal side of the contralateral flap. Next, the now outer fascial flap is laid over the inner flap, and a buttonhole is made to expose the umbilicus. The subcutaneous tissue of the inner flap is laterally undermined to gain extra distance for the suture of the outer flap over the inner flap. The subcutaneous tissue and skin are sutured in the midline, incorporating the umbilicus. RESULTS: In a 30-year period, 43 PBS patients underwent this procedure with good cosmetic and long-term functional results. CONCLUSION: This abdominoplasty technique is simple and presents good functional and cosmetic results in PBS patients.

27 years of experience with the comprehensive surgical treatment of prune belly syndrome.

INTRODUCTION: Prune belly syndrome (PBS) presents with three main features: abdominal wall flaccidity, urological abnormalities and cryptorchidism. As a result, urologists must consider the eventual repair of the abdominal wall flaccidity and urinary tract abnormalities, and the mandatory correction of cryptorchidism, as well as decide whether to perform the procedures in a single comprehensive approach or in multiple steps. OBJECTIVES: To report experiences with comprehensive surgical management of prune belly syndrome. MATERIAL AND METHODS: From 1987 to 2014, 46 children with PBS were submitted for comprehensive surgical treatment. According to individual needs, treatment aimed to correct the abdominal flaccidity, reconstruct the urinary tract, and perform bilateral orchiopexy and circumcision, which were performed in one procedure. Urinary tract reconstruction was indicated whenever pyelo-ureteral dilatation with evidence of significant stasis and/or vesicoureteral reflux was associated with recurrent urinary tract infections (UTI). Treatment for this cohort included: 44 abdominoplasties, 40 upper urinary tract reconstructions, 44 cystoplasties associated with three appendico-vesicostomies, 46 bilateral orchiopexies and 36 circumcisions. The median age at surgery was 16 months and children were followed for a median of 143 months. RESULTS: Abdominal appearance and tonus were improved in 90% of the children after the primary surgery and 100% after reoperation. Upper urinary tract reconstruction was performed in most children and long-term follow-up showed functional stabilization of the urinary tract in about 90% of the children, with progression to renal failure in 10%. Lower urinary tract reconstruction was performed in most children (95.6%); on late follow-up, continence was observed in 81% of them, while incontinence was present in 19% and usually associated with polyuria. Adequate bladder emptying was possible in most boys (82.6%), while the remaining required clean intermittent catheterization. Pre-operative UTI was present in 89.1% and urinary sepsis in 15.2%. Postoperatively, the incidence of laboratorial UTI was significantly reduced to 39.1%, while urinary sepsis was absent. Bilateral orchiopexy was performed in all children, with 85% of the testes becoming normal in size and well located in the scrotum. CONCLUSIONS: Comprehensive surgical treatment is feasible and has good long-term results. A considerable incidence of reoperations due to complications or progression of the disease was observed. The long-term results for reno-ureteral anatomy and function, bladder function, infection, testicular size and location, as well as abdominal aspect and tonus, show that comprehensive surgery is an adequate method for managing children with PBS.